Hybrid pulmonary artery plication followed by transcatheter pulmonary valve replacement: Comparison with surgical PVR

Objective/Background Historically, the sole option for patients with a dysfunctional native right ventricular outflow tract (RVOT) requiring re‐establishment of pulmonary competence has been surgical PVR. We sought to compare early outcomes of hybrid pulmonary valve replacement (PVR) combining surgical plication of the main pulmonary artery followed by transcatheter PVR, with a contemporary cohort of surgical PVR patients. Methods Retrospective chart analysis of all patients with a dilated native RVOT eligible for surgical PVR over 36 months was performed. The cohorts included patients with previous tetralogy of Fallot repair (n = 14), and previous intervention for congenital abnormality of the pulmonary valve (n = 7). Results Twenty‐one patients with a dysfunctional native RVOT met criteria for PVR; 8 using the hybrid procedure (group 1: age, 31.5 +/? 17.4 years) and 13 with cardiopulmonary bypass (CPB) (group 2: age, 31 +/? 18.4 years). Valve delivery was successful in all patients with no procedural mortality. Group 1 had a lesser requirement for blood products (P =< 0.001) and a trend toward shorter hospital stay and higher post‐operative hemoglobin. No patients in group 1 received inotropic support post‐operatively compared to 54% of patients in group 2. Mean follow‐up was 3.4 months for group 1 and 13.6 months for group 2 with the average peak gradient across the RVOT of 20.1 and 15.1 mm Hg respectively (P = 0.12), all with no more than mild PI. Conclusions Transcatheter hybrid PVR following RVOT plication provides a reasonable alternative to surgical PVR particularly in higher risk cohorts, reducing possible longer‐term consequences of repeated runs of CPB. © 2016 Wiley Periodicals, Inc.     

Postoperative and short‐term atrial tachyarrhythmia burdens after transcatheter vs surgical pulmonary valve replacement among congenital heart disease patients

Objective: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC‐) or surgical (S‐) pulmo‐ nary valve replacement (PVR).
Design/Setting: This was a retrospective observational study of patients who under‐ went PVR from 2010 to 2016 at UCLA Medical Center.
Patients: Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded.
Outcome Measures: The primary outcome was a time‐to‐event analysis of sustained AT. Sustained ATs were defined as focal AT, intra‐atrial reentrant tachycardia/atrial flutter, or AF lasting at least 30 seconds or terminating with cardioversion or anti‐ tachycardia pacing.
Results: Two hundred ninety‐seven patients (TC‐PVR, n = 168 and S‐PVR, n = 129) were included. During a median follow‐up of 1.2 years, nine events occurred in TC‐PVR group (5%) vs 23 events in S‐PVR group (18%). In the propensity adjusted models, the following factors were associated with significant risk of AT after PVR: history of AT, age at valve implantation, severe right atrial enlargement, and S‐PVR. In the secondary analysis, TC‐PVR was associated with lower adjusted risk of AT events in the postoperative epoch (first 30 days), adjusted IRR 0.31 (0.14‐0.97), P = .03, but similar risk in the short‐term epoch, adjusted IRR 0.64 (0.14‐2.94), P = .57.
Conclusion: There was an increased risk of AT in the first 30 days following S‐PVR compared to TC‐PVR. Additional factors associated with risk of AT events after PVR were a history of AT, age at valve implantation, and severe right atrial enlargement.     

Role of Surgery on Growth of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum: Mid-Term Results of Modified Right-Ventricular Overhauling Procedure

Objectives: To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve (TV) growth in patients with pulmonary atresia with intact ventricular septum (PAIVS). Methods: We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling (mRVoh) between 2008 and 2019 at two institutions. Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle, peeling off fibrotic endocardial tissue in the right ventricle (RV) cavity, surgical pulmonary valvotomy, and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass. The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh. Results: No mortalities were observed during a median follow-up of 3 years (interquartile range: 1.3–4.7 years) of follow-up were noted. mRVoh was performed at a median age of 163.5 days (range: 21–560 days), including seven neonates and two infants (<60 days). During follow-up, the median TV annular z-score increased significantly from −2.24 to −1.15 before and after mRVoh (p = 0.004). In ten patients with a prior history of percutaneous interventions for RV outflow tract (RVOT) widening at least 6 months before mRVoh, the TV annular z-score significantly changed during the period after mRVoh (−2.03 to −1.61, p = 0.028) compared with the period before mRVoh (−2.51 → –2.03, p = 0.575) after percutaneous intervention only. Conclusions: mRVoh in PAIVS patients was positively associated with TV annular growth, and it was more effective than percutaneous RVOT widening interventions without mRVoh.     

Hybrid transcatheter pulmonary valve replacement with a SAPIEN S3 valve after pulmonary artery banding via left lateral thoracotomy

For many patients with repaired congenital heart disease, the need for reintervention on dysfunctional right ventricular outflow tracts is pervasive. Many such patients are poor candidates for both transcatheter pulmonary valve replacement and cardiopulmonary bypass, and hybrid surgical and transcatheter procedures have evolved to meet this need. We present two cases of hybrid pulmonary valve replacement involving pulmonary artery band placement via left anterior thoracotomy followed by transvenous placement of a SAPIEN S3 valve without prestenting. This approach avoids cardiopulmonary bypass as well as redo sternotomy and will likely see an increase in utilization in the future.     

Long-Term Outcome and Risk Factor Analysis of Surgical Pulmonary Valve Replacement in Congenital Heart Disease

Objectives: To establish long-term outcome of surgical pulmonary valve replacement (PVR) in congenital heart disease (CHD) and to identify risk factors for overall mortality, operative mortality, and repetitive PVR. Methods: This is a retrospective study of 375 surgical PVR in 293 patients who underwent surgical PVR for CHD between January 2000 and May 2020. We only included patients with index PVR with previous open-heart surgery regardless of the number of PVRs. The previous surgical history of patients who underwent PVR during the study period was also included. Patients who underwent the Rastelli operation, and those who underwent single PVR without previous open-heart surgery were excluded. Results: The median age of the patients at the time of surgical PVR was 14.9 years (Interquartile range, IQR, 11.0–22.0). The median follow-up duration was 10.5 years (IQR, 5.5–14.8 years). There were 3 patients with operative mortality (1.0%) and 15 patients with overall mortality (5.1%). The survival rate was 95.1% over 20 years follow-up period. Multivariate analysis demonstrated that more than 3 times of previous open-heart surgeries before surgical PVR, older age at the first operation, longer cardiopulmonary bypass (CPB) time and longer intensive care unit (ICU) stay were predictors for overall mortality. Patients who underwent surgical PVR after more than 3 times of previous open-heart surgeries had significantly higher mortality than those who underwent open-heart surgeries less than 3 times (P < 0.001). Age younger than 10 years, male, multiple valve problems and longer ICU stay were significant predictors for repetitive PVR by multivariate analysis. Conclusions: Though surgical PVR has excellent long-term outcome, it should be performed with caution for those who previously underwent multiple open-heart surgeries, especially if patient received more than 3 times of open-heart surgeries.     

Outcomes After Transcatheter Reintervention for Dysfunction of a Previously Implanted Transcatheter Pulmonary Valve

ObjectivesThe aim of this analysis was to evaluate outcomes following transcatheter reintervention for degenerated transcatheter pulmonary valves (TPVs).BackgroundTPV replacement (TPVR) with the Melody valve demonstrated sustained relief of right ventricular outflow tract (RVOT) obstruction and pulmonary regurgitation.MethodsAll patients who underwent TPVR with a Melody valve as part of 3 Medtronic-sponsored prospective multicenter studies were included. Transcatheter reinterventions included balloon dilation of the previously implanted Melody valve, placement of a bare-metal stent within the implanted TPV, or placement of a new TPV in the RVOT (TPV-in-TPV). Indications for reintervention, decisions to reintervene, and the method of reintervention were at physician discretion. All patients provided written informed consent to participate in the trials, and each trial was approved by local or central Institutional Review Boards or ethics committees at participating sites.ResultsA total of 309 patients who underwent TPVR were discharged from the implantation hospitalization with Melody valves in place. Transcatheter reintervention on the TPV was performed in 46 patients. The first transcatheter reintervention consisted of TPV-in-TPV in 28 patients (median 6.9 years [quartile 1 to quartile 3: 5.2 to 7.8 years] after TPVR), simple balloon dilation of the implanted Melody valve in 17 (median 4.9 years [quartile 1 to quartile 3: 4.0 to 6.0 years] after TPVR), and bare-metal stent placement alone in 1 (4.4 years after TPVR). There were no major procedural complications. Overall, 4-year freedom from explant and from any later RVOT reintervention after the first reintervention were 83% and 60%, respectively. Freedom from repeat RVOT reintervention was longer in patients undergoing TPV-in-TPV than balloon dilation (71% vs. 46% at 4 years; p = 0.027).ConclusionsTPV-in-TPV can be an effective and durable treatment for Melody valve dysfunction. Although balloon dilation of the Melody valve was also acutely effective at reducing RVOT obstruction, the durability of this therapy was limited in this cohort compared with TPV-in-TPV.     

First human implant of the Alterra Adaptive PrestentTM: A new self‐expanding device designed to remodel the right ventricular outflow tract

Current balloon expandable transcatheter valves have limited applicability to patients with “native” right ventricular outflow tracts (RVOT), meaning those who have had previous surgery and are left with large, compliant, irregular RVOT. The Alterra Adaptive Prestent^TM is a self‐expanding, partially covered stent that was designed to internally reconfigure these types of RVOT, making them suitable for implantation of a commercially available balloon expandable heart valve, the SAPIEN 3. Herein, we describe the first human implant of this device.     

Acquired Coronary Artery Disease in Patients with Congenital Heart Disease: Issues in Diagnosis and Management

Objective: Acquired coronary artery disease, initially thought to rarely affect survivors of congenital heart disease, is increasingly recognized in this population, as these patients grow in age and numbers in the recent era. This study reports our experience with coronary artery disease in adults with congenital heart disease and discusses treatment issues and the existing literature. Methods: Retrospective review of all charts of adults with congenital heart disease and acquired coronary artery disease was performed. Patients’ clinical characteristics, diagnosis, risk factors, noninvasive and invasive imaging and management data were recorded. Results: Coronary artery disease was diagnosed at 35–70 of age in 17 patients out of a total of 1345 adult congenital heart disease patients followed. Congenital heart disease was moderate or complex in 5 patients (4 repaired Tetralogy of Fallot, 1 repaired ventricular septal defect) and simple unrepaired atrial septal defect diagnosed later in life before or after coronary artery disease identi- fication in 12 patients. Coronary artery disease symptoms were present in 12 patients (8 myocardial infarctions, 4 exercise intolerance), while all patients had 2–3 risk factors for coronary artery disease. Coronary angiography showed 1–3 vessel disease treated with percutaneous coronary intervention in 7 patients, coronary artery bypass graft in 2 patients and both in 2 patients. Patients received appropriate medical therapy and remained stable for 1–17 years, while 2 patients are awaiting surgical pulmonary valve replacement in the near future. Conclusion: Coronary artery disease can develop in adults with congenital heart disease later in life in the presence of traditional risk factors, and prevention, screening and treatment strategies should be applied in this population. Further studies are needed to fully elucidate the extent of this issue in this increasing and ageing population and to determine its optimal medical, interventional and surgical management.     

Bailout perventricular pulmonary valve implantation following failed percutaneous attempt using the Edwards Sapien transcatheter heart valve

Percutaneous pulmonary valve implantation has emerged as an attractive and less invasive alternative to open‐heart surgery. Nevertheless, major technical challenges and procedural complications may still occur. We report a 44‐year‐old woman with severe dextroscoliosis, and history of Tetralogy of Fallot repair, who underwent an attempt at percutaneous implantation of a 23‐mm Edwards SAPIEN transcatheter heart valve (THV) for recurrent stenosis of the right ventricle to pulmonary artery conduit. The procedure was complicated by valve migration into the right ventricle requiring emergent surgical removal using inflow‐occlusion technique to avoid cardiopulmonary bypass. A hybrid off‐pump perventricular approach was then used in the operating room to successfully implant a 26‐mm Edwards Sapien THV under fluoroscopic guidance. © 2010 Wiley‐Liss, Inc.     

Estimation of pulmonary vascular resistance: correlation between echocardiography and catheterization data in patients with congenital heart disease

Background: The ratio of peak tricuspid regurgitation velocity (TRV) and right ventricular outflow time–velocity integral (TVI RVOT) has been described as a good correlate of pulmonary vascular resistance (PVR). However, this method has not been well studied in congenital heart disease. Method: Twenty patients with post‐tricuspid shunt lesions who were planned to undergo cardiac catheterization were enrolled for the study. The ratio of TRV/TVI_RVOT was measured via transthoracic echocardiography and correlated with invasively derived PVR (PVR_CATH). PVR_CATH was measured by cardiac catheterization. Fick's principle was used to calculate the pulmonary blood flow and oxygen consumption was assumed. Linear regression analysis was done to find the correlation between TRV/TVI_RVOT and PVR_CATH. Results: There was a significant correlation between the two variables, r = 0.635(P = 0.003). Subgroup analysis revealed that this correlation was better at lower values of PVR_CATH (r = 0.817 for PVR < 6 Wood units (WU)) than higher values (r = 0.659 for PVR > 6 WU). TRV/TVI_RVOT ratio of greater than 0.145 predicted with 80% sensitivity and specificity a PVR > 6 WU. Conclusions: There is modest correlation between TRV/TVI_RVOT ratio and invasively derived PVR in congenital shunt lesions, especially in PVR < 6 WU. TRV/TVI_RVOT ratio could be useful in identifying patients with congenital shunts whose PVR is likely to be <6 WU, and hence, do not need cardiac catheterization. (Echocardiography 2012;29:478‐483)     

Hand‐held echocardiography in children with hypoplastic left heart syndrome

Background: When performed by cardiologists, hand‐held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.
Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the same day as standard echocardiography (SE). Using 4‐point scales, bedside HHE assessment of RV systolic function and TR were compared with blinded assessment of offline SE images. Concordance correlation coefficient (CCC) was used to evaluate agreement.
Results: Thirty‐two HHEs were performed on 15 subjects (Stage I: n = 17 and Stage II: n = 15). Median subject age was 3.4 months (14 days‐4.2 years). Median weight was 5.9 kg (2.6‐15.4 kg). Bedside HHE assessment of RV systolic function and TR severity had substantial agreement with SE (CCC = 0.80, CCC = 0.74, respectively; P < .001). HHE sensitivity and specificity for any grade of depressed RV systolic function were 100% and 92%, respectively, and were 94% and 88% for moderate or greater TR, respectively. Average HHE scan time was 238 seconds.
Conclusions: HHE offers a rapid, bedside tool for pediatric cardiologists to detect RV systolic dysfunction and hemodynamically significant TR in HLHS.     

Use of the GORE^® DrySeal Flex Introducer Sheath to Facilitate Implantation of the Transcatheter Venus <i>P</i>-valve

Objectives: We report our experience of using the 65 cm large diameter GORE^® DrySeal Flex Introducer sheath to facilitate transcatheter implantation of the Venus P-valve in the pulmonary position. Background: Transcatheter implantation of pulmonary valves can be difficult due to rigidity of the valve delivery system or the anatomy of the RVOT and pulmonary artery bifurcation and the risk of iatrogenic damage to the tricuspid valve support apparatus. Using long sheaths to pass and protect the tricuspid valve may facilitate the procedure. Methods: Multi-centre registry of patients who underwent transcatheter pulmonary valve implantation of the Venus P-valve using the GORE^® DrySeal Flex introducer sheath to facilitate passage of the valve to the right ventricular outflow tract. Procedural success, time to valve implantation and radiation safety parameters were analyzed. These data were compared to a control group of subjects treated between July 2014 and May 2016 with the same valve but without the use of GORE^® DrySeal. Results: Between December 2016 and September 2018, the Venus P-valve was successfully deployed through the GORE^® DrySeal in 12 patients. There were no procedure-related complications. As a control group, 10 subjects treated between July 2014 and May 2016 were included. Total procedure time was significantly shorter in the GORE^® DrySeal group compared to the control group 96 ± 27 min vs. 164 ± 12 min (p < 0.001). Total screening time was significantly shorter in the GORE^® DrySeal group (24 ± 11 min) when compared with the control group (32 ± 14 min, p < 0.001). Conclusions: We describe a modification to the previously described techniques of implanting the Venus P-valve in the pulmonary position after surgical repair of congenital heart disease. In our experience, the GORE^® DrySeal sheath has considerably facilitated the procedure and reduced the potential risks.     

Outcomes of Self-Expanding Transcatheter Pulmonary Valves: Extended Follow-Up of a Prospective Trial

Background: The Venus-P valve was the first self-expanding valve used world-wide for transcatheter pulmonary valve replacement (TPVR) in patients with severe pulmonary regurgitation (PR). We intended to report the extended follow-up results from the prospective trial (No. NCT02590679). Methods: A total of 38 patients with severe PR (mean age 24.2 ± 13.2) were included. Follow-up data were obtained after implanted at 1, 6, and 12 months and yearly after. The frame geometry was assessed on post-implant computer tomography (CT) scanning by calculating the non-circularity [circularity ratio (minimum diameter/maximum diameter) < 0.9] and under-expansion [expansion ratio (derived external valve area/nominal external valve area) < 0.9). Adverse events (all-cause mortality, reintervention, valve dysfunction, stent fracture and endocarditis) were recorded. Results: All valves were implanted successfully with normal function at discharge. Geometric CT analysis showed under-expanded valve was detected in 22 patients (63%) and non-circular valve was seen in 16 patients (46%). During a median follow-up of 4.8 years (range 0.3–8.1), there were 1 death and 1 surgical explant, both resulting from endocarditis. Five-year freedom from valve dysfunction and stent fracture were 84.8% (95%CI 74.8–94.7) and 83.5% (95%CI 73.8–93.2). Endocarditis occurred in 3 patients at a median time of 7 months. Stent fracture was more common in patients with non-circularity stents. Conclusion: TPVR using Venus-P valve is associated with favorable outcomes at 5 years. Non-circular shapes in the valve level may have a higher risk of stent fracture.     

Hybrid approach for harmony transcatheter pulmonary valve replacement

The Harmony™ Transcatheter Pulmonary Valve (Medtronic) was recently approved by the Food and Drug Administration for transcatheter pulmonary valve replacement in native right ventricular outflow tracts. Despite this milestone, some patients have main pulmonary arteries that are severely dilated and continue to require surgical pulmonary valve replacement. The hybrid approach combines surgical creation of a landing zone, transcatheter valve deployment, and suture stabilization of the implanted valve. In this case series, we report the first use of a hybrid approach for Harmony™ transcatheter pulmonary valve replacement. Two cases are reported with varying approaches for surgical creation of a landing zone followed by successful placement of a Harmony™ valve.     

Immediate and Long-Term Results of Transcatheter Closure of Patent Ductus Arteriosus—Comparison of Two Decades before and after Change in Antibiotic Infective Endocarditis Prophylaxis Guidelines

Objectives: To determine immediate and long-term follow-up of transcatheter closure of patent ductus arteriosus (PDA) in children. Background: National antibiotic prophylaxis (AP) guideline for infective endocarditis changed after 2009, the effect on practice of PDA closure is unknown. Methods: Observational single center study analyzing follow-up of PDA closure comparing two time periods before (2002–2009) and after (2010–2019) changes in AP guideline. Results: 332 patients (68.1% female), median (interquartile range) age 3.0 years (1.5–5.7) and body weight 14.0 kg (10.0–19.3), were enrolled. PDA morphology was conical type A (50.3%), window type B (1.2%), tubular type C (40.1%), complex type D (2.1%), elongated type E (0.9%) and other (5.4%). Minimal PDA diameter and length were 1.9 mm (1.3–2.5) and 8.0 mm (6.2–10.2). PDA was closed using coils (56.3%), Amplatzer Duct Occluders (41.9%) and others (1.8%). Complete closure rate was 61.1% at catheter intervention, 72.3% on day 1, 87.7% after 6 months and 98.4% at last follow-up on echocardiography. Moderate complication rate (severity level 3) was 4.2% and major complication rate (severity level 4) 0.3%, with no catastrophic complications (severity level 5). Annual PDA closure rate declined in the second time period (22.6/year vs. 15.5/ year, p = 0.018), PDA size increased (1.6 mm vs. 2.0 mm, p = 0.002) and proportion of coils decreased (72.4% vs. 37.1%, p < 0.001). Conclusions: Interventional closure of PDA is associated with excellent closure rates during follow-up (>98%) and only a small number of complications leading to reintervention or surgery. Change in AP guidelines changed indication for and practice of PDA closure.     

Retrograde transcatheter device closure of a complex paravalvular leak after bioprosthetic pulmonary valve replacement in a pediatric patient

We report a case of retrograde transcatheter device closure of a complex paravalvular leak (PVL) after bioprosthetic pulmonary valve replacement (PVR) in a 13‐year‐old patient with congenital pulmonary valve stenosis. There are prior reports of pulmonary PVL closure after PVR in adults (Seery and Slack, Congenit Heart Dis 2014;9:E19–F22), but indications for and technical considerations in PVL closure after bioprosthetic PVR, particularly in children, are not well defined. © 2015 Wiley Periodicals, Inc.     

Longitudinal Health-Related Quality of Life Assessment in Children with Congenital Heart Disease

Objective: Health-related quality of life (HRQoL) has become an important outcome measure for patients with congenital heart disease (CHD). The aim of this study was to evaluate the natural course of HRQoL from longitudinal assessment in children with CHD. Patients and Methods: From July 2014 to February 2020 this longitudinal study recruited 317 children with CHD (113 girls, 35.6%) aged 6 to 18 years (11.6 ± 2.9 years). HRQoL was assessed with the generic, self-reported and age-adapted KINDL^® questionnaire. During a mean follow-up period of 2.2 ± 1.3 years, 195 patients had one HRQoL reassessment, 70 two, 40 three and 12 patients four or more re-assessment, respective. Results: Overall HRQoL at baseline was 78.7 ± 9.3. During follow-up there were no changes in HRQoL over time (0.03 [–0.01–0.07]; p = 0.195). In a linear mixed model neither CHD severity, the diagnostic subgroup, age, BMI, surgical history nor gender could be linked to a change in HRQoL during the follow-up time. Only children with higher age baseline (–0.48 [–0.85––0.11]; p = 0.010) had lower HRQoL. Same trend was seen for BMI (–0.19 [–0.41–0.03]; p = 0.099). Conclusion: Older children with CHD have significantly worse HRQoL, but they evolve similarly to younger children over time. Since no demographic or clinical variable could be linked to the course of HRQoL, it seems that individual HRQoL courses are not predictable and routine HRQoL evaluations seem to be necessary for acute decision making in clinical practice.     

Chronotropic Response and Pulmonary Function are Associated with Exercise Performance in Children and Adolescents with Repaired Tetralogy of Fallot Independent of Cardiac Function

Objective: The determinants of exercise capacity in repaired tetralogy of Fallot (rTOF) are multifactorial and remain incompletely understood. This study sought to evaluate the association of chronotropic response with exercise parameters and investigate the determinants of heart rate reserve (HRR) in a cohort of children and adolescents with rTOF. Design: We retrospectively analyzed patients with rTOF, age 8–18 years, who underwent cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) for research purposes. Linear regression models were performed to test associations among clinical, CMR and CPET parameters. Outcomes included percent-predicted maximum VO₂ (%mVO₂) and HRR. Results: A total of 148 patients were included (mean age 12.3 ± 3.1 years). The majority of patients had TOF with pulmonary stenosis (80%) and underwent transannular patch TOF repair (78%). Median age at surgical repair was 4.2 months (IQR 1.2, 8.4). There was preserved RV ejection fraction (60.4 ± 8.3%) and moderate pulmonary insufficiency (regurgitant fraction 35.2 ± 16.6 %). On CPET, %mVO₂ was overall diminished (76.5 ± 17.9 %), and % predicted forced vital capacity (FVC) was diminished on spirometry. HRR, FVC and ability to reach maximum effort were independently associated with greater %mVO₂. FVC, net forward flow in the main pulmonary artery/m² , and reaching maximum effort were associated with greater HRR, independently of RV volume, degree of PI and RV ejection fraction. Conclusions: In patients with rTOF, HRR and pulmonary function (FVC) are more important contributors to exercise performance than right ventricular function. Interventions to improve chronotropic health and pulmonary function should be explored.     

Quality of Life in Congenital Heart Disease Patients According to Their Anatomical and Physiological Classification

Background: Living well is as important as living longer. The objective of this study is to assess quality of life (QoL) in congenital heart disease (CHD) according to current AHA/ACC anatomical and physiological classifi- cation. Methods: Cross-sectional study examining the World Health Organization QoL Bref questionnaire (WHOQoL-Bref) in consecutive outpatient CHD patients from a single unit. Results: 191 CHD patients were studied. Median age was 28 ± 13 years and 59% were male. 44 (23%), 115 (60%) and 33 (17%) CHD patients showed mild, moderate and great anatomical defects respectively while 69 (36%) patients were in physiological Stage A, 27 (14%) in Stage B, 84 (44%) in Stage C and 11 (6%) in Stage D. No significant differences were seen in relation the anatomical classification and the different sections of the WHOQoL-Bref questionnaire. CHD patients in Stages C and D had significant lower physical domain scores than patients in the Stage A (p < 0.05). However, no significant differences were seen in the psychological, social relationships and environmental domains. The binary logistic regression analysis showed that having a higher educational level was a protective factor [OR 0.32 (95% CI, 0.12–0.87), p = 0.026] while being married or cohabit was a risk factor [OR 3.46 (95% CI, 1.13–10.63), p = 0.030] for having a worse rated QoL. Meanwhile, having a worse functional class (NYHA ≥2) [OR 3.44 (95% CI, 1.20–9.81), p = 0.021] was associated with dissatisfaction with health. Conclusion: Patients with advanced physiological stages scored lower on the physiological domain. No statistical significance was seen, according to the anatomical and physiological classification, in the psychological, social relationship and environmental domains.     

What Is the Relation between Aerobic Capacity and Physical Activity Level in Adults with Congenital Heart Disease?

Background: Aerobic capacity (AC) in adults with congenital heart disease (CHD) is often reduced, mainly due to low confidence levels towards physical activity (PA). The main objective of this study was to estimate the association between PA level and AC (measured as peak of oxygen consumption, VO_2peak) in adults with CHD. Methods: A total of 183 individuals (83 women and 100 men; mean (SD) age 36.9 (11.0) years old) from Vall d’Hebron Hospital, Barcelona-Spain in 2019, participated in this cross-sectional study. The AC was assessed by cardiopulmonary exercise testing (CPET) using a treadmill ramp protocol. Considering values of metabolic equivalent of task (MET, MET-min·week^-1 ) obtained by the short International Physical Activity Questionnaire (IPAQ), participants were divided into three categories of PA: health-enhancing PA (HEPA), minimally active, and inactive. Results: Median (SD) PA was 2737.2 (2835.7) MET-min·week^-1 , with 60 participants (32.8%) reporting HEPA, 91 (49.7%) minimally active, and 32 (17.5%) inactivity. Participants demonstrated a mean VO_2peak of 28.9 (8.8) mL·Kg^-1 ·min^-1 , showing AC values on average 13% lower than expected in a healthy population. Overall, PA and AC were positively associated. Adjusting for sex and age, an increase of 1000 MET-min·week^-1 was associated with an increase in VO_2peak of 0.8 units (95% CI 0.4–1.2; p < 0.001). There were no differences in the degree of increase between sexes (p = 0.427). Conclusion: These findings suggest that an increase in PA in patients with CHD significantly improves their AC, and hence, could be recommended when the goal is to improve their physical condition.